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• Hereditary Periodic Fever Syndromes
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Hereditary periodic fever syndromes are hereditary disorders characterized by recurrent fever and other symptoms that are not explained by other causes.

Most patients develop symptoms during childhood; < 10% develop symptoms after age 18. Disorders best characterized are

• Familial Mediterranean fever
• Hyper-IgD syndrome
• Tumor necrosis factor (TNF) receptor–associated periodic syndrome

Others include

• The hereditary cryopyrinopathies: Familial cold autoinflammatory syndrome, Muckle-Wells syndrome, and neonatal-onset multisystem inflammatory disease (NOMID)
• PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome
• PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome

Last full review/revision January 2009 by Stephen E. Goldfinger, MD

Content last modified January 2009